Hemophilia B is a serious hemostasis disorder due to mutations of the factor IX gene in the X chromosome. Gene therapy has gained momentum in recent years as a therapeutic option for hemophilia B. In hemophilia, reconstitution with a mere 1–2% of the clotting factor improves the quality of life, while 5–20% suffices to ameliorate the bleeding disorder. A paper by Guan et al (2016) in this issue...

conclusions the diagnostics and management of infections with hepatotropic viruses, particularly hbv, are neglected in hemophilic patients. all patients with coagulation disorders and a history of exposure to non-inactivated blood products should be screened for blood-borne infections. the prevalence of other potentially blood-borne viral infections exhibited a pattern similar to that observed ...

Background: Heterogeneous mutations in the human coagulation factor IX gene lead to an X-linked recessive bleeding disorder known as hemophilia B. The disease is distributed worldwide with no ethnic or geographical priority. Materials and Methods: The aim of this study was to characterize the factor IX gene mutations in 28 unrelated Iranian hemophilia B patients. Polymerase chain reaction (PCR)...

Aminoglycoside antibiotics exhibit their bactericidal effect by interfering with normal ribosomal activity. In this pilot study, we have evaluated the effect of the aminoglycoside antibiotic gentamicin on the factor VIII (FVIII) and IX levels of severe hemophiliacs with known nonsense mutations. Five patients were enrolled and each patient was given 3 consecutive days of gentamicin at a dose of...

Background. Patients with hemophilia are protected from thrombosis by a deficiency of one coagulation factors, therefore thrombotic complications rare in them. Currently, few descriptions spontaneous venous adult hemophiliacs can be found the literature. Even fewer data thromboembolic reported pediatric practice. At present, no clear recommendations given for management children hemophilia, whi...

BACKGROUND Pain, as a crucial subsequence of joint hemorrhages in hemophilia patients, is chronic, debilitating, and distracting. This study aimed to describe and interpret pain experiences of hemophilia patients in their lives. METHODS This qualitative study with hermeneutic phenomenological approach was conducted on fourteen hemophilia patients who had been referred to a hemophilia center a...

background: hemophilia b is an x-linked hereditary disorder of blood coagulation system which is caused by factor ix (fix) deficiency. factor ix is a plasma glycoprotein that participates in the coagulation process leading to the generation of fibrin. replacement of factor ix with plasma-derived or recombinant factor ix is the conventional treatment for hemophilia b to raise the factor ix level...

conclusions based on our results, it seems that there were no cases of obi among chronic hcv-infected patients with thalassemia and bleeding disorders, particularly hemophilia. however, to improve decisions concerning obi screening, especially in transfusion centers, and concerning the use of comprehensive screening methods, more original studies with more precise laboratory techniques and larg...

Hemophilia A and B are the most common of the severe bleeding disorders. The present article focuses on the practical aspects of the management of neonates and children diagnosed with hemophilia, and is based on questions frequently posed to paediatric hematologists. It highlights the importance of early diagnosis, the principle of early intervention and the role of comprehensive care hemophili...

Abstract In Coronavirus disease 2019 (COVID-19) subjects, recent evidence suggests the presence of unique coagulation abnormalities. this study, we performed clot waveform analyses to investigate whether specific modulations are observed in COVID-19 subjects. We analyzed second derivative absorbance routine APTT tests using an ACL-TOP system. high frequencies abnormal patterns second-derivative...